ILD in Sjögren Disease, Mixed Connective Tissue Disease, or SLE — What to Do When First-Line Immunosuppressive Therapy Has Not Achieved Stabilisation

Clinical Scenario

This protocol is for patients with Sjögren disease (SjD), mixed connective tissue disease (MCTD), or systemic lupus erythematosus (SLE) who have interstitial lung disease and are on, or have completed, a first course of immunosuppressive treatment. Immunosuppressive treatment is recommended for ILD arising in the context of these connective tissue diseases.

When First-Line Treatment Falls Short

This protocol addresses the scenario in which prior immunosuppressive therapy — which may have included mycophenolate, azathioprine, glucocorticoids, rituximab, cyclophosphamide, or combinations thereof — has failed to achieve improvement or stabilisation of FVC% and DLCO%. Non-achievement of these spirometric goals is the trigger for escalation to this next treatment step.

Next-Step Approach (Partial Overview)

The protocol involves modifying the immunosuppressive regimen — whether by adding to or switching the current therapy — with a distinct additional consideration for patients whose course is consistent with progressive pulmonary fibrosis. The full agent selection, sequencing, and decision algorithm are available in the complete protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1183/13993003.02533-2024

We suggest using immunosuppressive treatment in patients with SjD, MCTD and SLE-ILD (conditional recommendation, very low certainty of evidence; no studies included).

If on mono or combination treatment, add or switch immunosuppressant.

If progressive pulmonary fibrosis, add nintedanib.

View source ↗